Tariq Faiza, Eilbert Wesley
University of Illinois, College of Medicine, Department of Emergency Medicine, Chicago, Illinois.
Clin Pract Cases Emerg Med. 2024 Aug;8(3):243-245. doi: 10.5811/cpcem.7223.
Spontaneous intracranial hypotension (SIH) is an uncommon and frequently misdiagnosed condition characterized by a lower-than-normal volume of cerebrospinal fluid (CSF) caused by leakage of CSF through the dural membrane. The primary manifestation of SIH is an orthostatic headache, which is frequently accompanied by nausea and vomiting. Patients with connective tissue disorders are at increased risk for spontaneous CSF leaks due to the structural weakness of their dural membranes.
An 18-year-old woman with no reported past medical history presented to the emergency department with 10 days of a bifrontal headache that was orthostatic in nature with associated nausea and vomiting. She was noted to have several marfanoid features on physical examination. Spontaneous intracranial hypotension was ultimately diagnosed and treated successfully with an epidural blood patch. Subsequent genetic testing revealed a diagnosis of Marfan syndrome.
Spontaneous intracranial hypotension is an uncommon cause of headache. Individuals with connective tissue disorders such as Marfan syndrome are at increased risk for SIH. Knowledge of the relationship between these two conditions allows for a more rapid diagnosis of SIH.
自发性颅内低压（SIH）是一种罕见且常被误诊的疾病，其特征是由于脑脊液（CSF）通过硬脑膜渗漏导致脑脊液量低于正常水平。SIH的主要表现是体位性头痛，常伴有恶心和呕吐。结缔组织疾病患者由于其硬脑膜结构薄弱，发生自发性脑脊液漏的风险增加。
一名18岁女性，既往无病史，因前额双侧头痛10天就诊于急诊科，头痛为体位性，伴有恶心和呕吐。体格检查发现她有一些类马凡氏特征。最终诊断为自发性颅内低压，并通过硬膜外血贴成功治疗。随后的基因检测显示诊断为马凡综合征。
自发性颅内低压是一种罕见的头痛病因。患有马凡综合征等结缔组织疾病的个体发生SIH的风险增加。了解这两种疾病之间的关系有助于更快速地诊断SIH。
